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Key Documents

S2944

Sigma-Aldrich

Anti-SMN antibody, Mouse monoclonal

clone 2B1, purified from hybridoma cell culture

Sinonimo/i:

Anti-Survival of Motor Neurons

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About This Item

Numero MDL:
Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

mouse

Livello qualitativo

Coniugato

unconjugated

Forma dell’anticorpo

purified from hybridoma cell culture

Tipo di anticorpo

primary antibodies

Clone

2B1, monoclonal

Forma fisica

buffered aqueous solution

Reattività contro le specie

Xenopus, human, mouse

Concentrazione

~2 mg/mL

tecniche

microarray: suitable
western blot: 2-4 μg/mL using A431 cell extract

Isotipo

IgG1

Condizioni di spedizione

dry ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

Descrizione generale

Mouse monoclonal clone 2B1 anti-SMN antibody recognizes human, mouse, and Xenopus survival of motor neurons proteins.

Specificità

The antibody recognizes human, mouse, and Xenopus SMN.

Immunogeno

recombinant human SMN

Applicazioni

Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Enzyme-linked immunosorbent assay (1 paper)
Mouse monoclonal clone 2B1 anti-SMN antibody is an important tool for studying the role of the survival of motor neurons protein in nuclear processes and spinal muscular atrophy (SMA). It may be used in immunoblotting (~35 kDa), immunoprecipitation, and immunocytochemistry.

Azioni biochim/fisiol

Survival of Motor Neurons (SMN) complex is important in various biological processes, such as assembly and restructuring of spliceosomal small nuclear ribonucleoproteins (snRNPs), pre mRNA splicing and transcription. Spinal muscular atrophy (SMA) is caused by reduced expression or mutations in the survival of motor neurons (SMN) protein. Deletion or mutation in the telomeric copy (SMN1) causes the SMA phenotype. The severity of SMA is in direct correlation with the expression level of the SMN protein, either from the SMN1 gene or a different spliced form of SMN from the SMN2 gene. The SMN complex interacts with various protein substrates such as Sm and Lsm proteins of the spliceosomal snRNPs, fibrillarin, GAR1, RNA helicase A, the human hnRNP proteins (hnRNPQ, U and R), coilin and p53.

Stato fisico

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15mM sodium azide.

Stoccaggio e stabilità

For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in "frostfree" freezers, is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.

Esclusione di responsabilità

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Codice della classe di stoccaggio

12 - Non Combustible Liquids

Classe di pericolosità dell'acqua (WGK)

WGK 2

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable


Certificati d'analisi (COA)

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Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?
Burghes AHM and Beattie CE
Nature Reviews. Neuroscience, 10(8), 597-597 (2009)
Dione T Kobayashi et al.
PloS one, 6(8), e24269-e24269 (2011-09-10)
Genetic defects leading to the reduction of the survival motor neuron protein (SMN) are a causal factor for Spinal Muscular Atrophy (SMA). While there are a number of therapies under evaluation as potential treatments for SMA, there is a critical
Q Liu et al.
The EMBO journal, 15(14), 3555-3565 (1996-07-15)
Spinal muscular atrophy (SMA) is a common, often fatal, autosomal recessive disease leading to progressive muscle wasting and paralysis as a result of degeneration of anterior horn cells of the spinal cord. A gene termed survival of motor neurons (SMN)
Thomas O Crawford et al.
PloS one, 7(4), e33572-e33572 (2012-05-05)
The universal presence of a gene (SMN2) nearly identical to the mutated SMN1 gene responsible for Spinal Muscular Atrophy (SMA) has proved an enticing incentive to therapeutics development. Early disappointments from putative SMN-enhancing agent clinical trials have increased interest in
Sergey Paushkin et al.
Current opinion in cell biology, 14(3), 305-312 (2002-06-18)
Spinal muscular atrophy is a common, often lethal, neurodegenerative disease that results from low levels of, or loss-of-function mutations in, the SMN (survival of motor neurons) protein. SMN oligomerizes and forms a stable complex with five additional proteins: Gemins 2-6.

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